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Other Malignant Conditions Affecting Musculoskeletal System
2. Understand the Diagnosis and Management of other malignant conditions, such as myeloma and other haematological conditions, that can affect the musculoskeletal system 'Myeloma' DIAGNOSIS For formal diagnosis, 2/3 of these key features should be present: *Paraproteinaemia/Bence Jones Protein *Radiological Evidence of lytic bone lesions - X-ray *An increase in bone marrow plasma cells - from bone marrow aspirate To define the myeloma as 'symptomatic', evidence of end organ failure should be present e.g. *anaemia, lytic bone lesions, renal impairment, hypercalcaemia, recurrent infection. Clinical suspicion of myeloma should be raised if an elderly patient presents with: *Bone pain *Symptoms of anaemia *Recurrent infections *Symptoms of renal failure *Symptoms of hypercalcaemia MANAGEMENT Median survival of myelome is 5 years, with good supportive care and chemotherapy some patients are now surviving for up to ten years; with young patients receiving aggressive treatment potentially surviving even longer. SUPPORTIVE THERAPY *Correct anaemia *Infections - annual 'flu vaccination, treat bacterial infection promptly with antibiotics *Prevention of pathological fractures with surgical intervention (kyphoplasty) and pinning of lytic lesions. *Treatment of pathological spinal fractures with kyphoplasty and vertebroplasty may be useful. SPECIFIC THERAPY *Myeloma remains incurable *Drugs used to prolong survival include prednisolone/dexamethasone, thalidomide/lenalidomide, alkylating agents such as melphalan, and a proteosome inhibitor called bortezomib. 'Primary Lymphoma of Bone' DIAGNOSIS Clinically, the majority of patients present with bone pain which is not relieved with rest. A palpable mass is present in 50% of cases. Swelling, pathologic fracture, and systemic malignant symptoms may also be present. Histological diagnosis is obtained via biopsy (either percutaneous or open).Diagnosis must also include a thorough history and examination to confirm the lymphoma is limited to the bone. Imaging - CT characterises thebone lesion, MRI is used to assess the extent of soft tissue involvement. PET scan identifies active bone sites. Plain X-rays may show 'medullary mottling'. MANAGMENT Management is dependent on staging. The staging criteria for Non-Hogkin's lymphoma should be used in any lymphoma patient with bone involvement. Primary Bone Lymphomas are classified according to the Ann-Arbor staging criteria. *IE is confined to one bony lesion *IIE is defined as one bony lesion with involvement of local regional lymph nodes *IV is defines as multiple lesions confined exclusively to bone The mainstay of management is chemotherapy, with or without radiotherapy. Surgery is generally limited to diagnotic biopsy and stabilisation of pathological fractures. 5- and 10- year survivals are 58% and 45% respectively. 'Leukaemia' DIAGNOSIS Acute childhood leukaemia often presents as arthritis. Clinical pictures include sudden onset of pain and fever in an otherwise healthy indiviual, and migratory arthritis. Affected joints are swollen and warm, with a decreased range of movement. Pathological fractures may occur. Diagnosis is made on the results of haematological investigation. Results depend on the type of leukaemia the individual is suffering from. MANAGEMENT *Chemotherapy *Supportive therapy 'Ewing's Sarcoma' DIAGNOSIS *Ewing's typically presents in long bone diaphyses and limb girdles of adolescents. *Radiology shows bone destruction, with conentric "onion ring" layers of new bone formation *MRI helps clarify extent of soft tissue involvement MANAGEMENT Chemotherapy, Bone resection, and Limb salvage surgery/radiotherapy cures ~50%. Metastases at diagnosis are a poor prognostic factor.